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Clinical and Experimental Rheumatology Sep 2022This review highlights publications on different aspects of Behçet's syndrome (BS) that appeared in 2021 and provides a critical view. These publications include works... (Review)
Review
This review highlights publications on different aspects of Behçet's syndrome (BS) that appeared in 2021 and provides a critical view. These publications include works on the epidemiology of BS across different continents, newly developed instruments to assess damage in BS, studies highlighting the immunopathogenesis, genetics and epigenetic factors, histopathology of the pathergy lesion, clinical and imaging aspects of vascular involvement, and safety and efficacy of therapeutic agents including tocilizumab, apremilast and direct oral anticoagulants.
Topics: Anticoagulants; Behcet Syndrome; Humans
PubMed: 35894066
DOI: 10.55563/clinexprheumatol/h4dkrs -
Clinical and Experimental Rheumatology 2020Behçet's syndrome is a variable vessel vasculitis with multi-system involvement that shows important heterogeneity among patients regarding demographic features, organ... (Review)
Review
Behçet's syndrome is a variable vessel vasculitis with multi-system involvement that shows important heterogeneity among patients regarding demographic features, organ manifestations, frequency and severity of relapses, disease course, response to treatment and prognosis. This heterogeneity has made it difficult to interpret and compare the results of studies, to standardise disease assessment and to develop management strategies. Several new studies have been published during the previous year exploring the epidemiology, pathogenesis, clinical manifestations, diagnosis, and management of Behçet's syndrome. The aim of this review is to provide an overview of the most relevant recent research in Behçet's syndrome from a critical perspective.
Topics: Behcet Syndrome; Disease Progression; Humans; Prognosis; Recurrence
PubMed: 33331271
DOI: No ID Found -
Clinical and Experimental Rheumatology Oct 2023This critical review of studies on Behçet's syndrome published during 2022 includes studies on epidemiology, patients' perspective, pathogenesis, diagnosis, clinical... (Review)
Review
This critical review of studies on Behçet's syndrome published during 2022 includes studies on epidemiology, patients' perspective, pathogenesis, diagnosis, clinical features and management. Studies on pathogenesis included potential biomarkers mostly related to macrophages, neutrophil and cytokine balance, new GWAS and polymorphism studies, and studies on miRNAs and long non-coding RNAs. Clinical studies showed that application of pneumococcal vaccine to the prick site increased the sensitivity and specificity of the pathergy test and the prevalence of AA amyloidosis had decreased over the years. Studies on management indicated that more data are needed to understand the effect of apremilast on BS manifestations other than oral ulcers, and new BS manifestations may develop during treatment with infliximab. Other biologics and Jak inhibitors might be an option for patients who are refractory to TNF-α inhibitors. Moreover, endovascular repair of arterial aneurysms might be an alternative to open surgery.
Topics: Humans; Behcet Syndrome; Infliximab; Tumor Necrosis Factor-alpha; Aneurysm; Sensitivity and Specificity; Tumor Necrosis Factor Inhibitors
PubMed: 37877363
DOI: 10.55563/clinexprheumatol/7kdo9x -
Current Rheumatology Reports May 2021To assess current management of Behcet's disease (BD). Controversies on therapeutic approaches to different manifestations, whether conventional immunosuppressives (IS)... (Review)
Review
PURPOSE OF REVIEW
To assess current management of Behcet's disease (BD). Controversies on therapeutic approaches to different manifestations, whether conventional immunosuppressives (IS) or biologic agents, should be chosen, and options for refractory disease are discussed.
RECENT FINDINGS
Glucocorticoids are still the main agents for remission-induction and azathioprine the first-line conventional IS in maintenance phase to prevent relapses of major organ involvement. Apremilast is shown to be a safe and effective option approved by the FDA for oral ulcers. Large case series confirmed the efficacy and safety of TNFα inhibitors and Interferon-α. Promising results are observed with IL-1 inhibitors, ustekinumab, secukinumab, and tocilizumab for refractory BD. Although both conventional IS and biologic agents are effectively used to suppress inflammation in BD, there is still an unmet need for clear therapeutic strategies in the management for different manifestations. Further controlled studies with new biologic agents, anticoagulants and the benefit of concomitant IS usage with biologics are needed to optimize the management of BD.
Topics: Anticoagulants; Azathioprine; Behcet Syndrome; Glucocorticoids; Humans; Immunosuppressive Agents; Remission Induction
PubMed: 34014377
DOI: 10.1007/s11926-021-01011-z -
American Journal of Ophthalmology Aug 2021To determine classification criteria for Behçet disease uveitis. (Comparative Study)
Comparative Study
PURPOSE
To determine classification criteria for Behçet disease uveitis.
DESIGN
Machine learning of cases with Behçet disease and 5 other panuveitides.
METHODS
Cases of panuveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated on the validation set.
RESULTS
One thousand twelve cases of panuveitides, including 194 cases of Behçet disease with uveitis, were evaluated by machine learning. The overall accuracy for panuveitides was 96.3% in the training set and 94.0% in the validation set (95% confidence interval 89.0, 96.8). Key criteria for Behçet disease uveitis were a diagnosis of Behçet disease using the International Study Group for Behçet Disease criteria and a compatible uveitis, including (1) anterior uveitis; (2) anterior chamber and vitreous inflammation; (3) posterior uveitis with retinal vasculitis and/or focal infiltrates; or (4) panuveitis with retinal vasculitis and/or focal infiltrates. The misclassification rates for Behçet disease uveitis were 0.6% in the training set and 0% in the validation set, respectively.
CONCLUSIONS
The criteria for Behçet disease uveitis had a low misclassification rate and seemed to perform sufficiently well for use in clinical and translational research.
Topics: Adult; Behcet Syndrome; Consensus; Female; Humans; Machine Learning; Male; Retrospective Studies; Uveitis; Young Adult
PubMed: 33845008
DOI: 10.1016/j.ajo.2021.03.058 -
Seminars in Arthritis and Rheumatism Feb 2022Behçet's disease (BD) is an auto-inflammatory disease, primarily characterized by recurrent painful mucocutaneous ulcerations. (Review)
Review
INTRODUCTION
Behçet's disease (BD) is an auto-inflammatory disease, primarily characterized by recurrent painful mucocutaneous ulcerations.
METHODS
A literature search was performed to write a narrative review into the pathogenesis and current treatment options of BD.
RESULTS
The pathogenesis of BD remains to be elucidated, but is considered a genetically primed disease in which an external trigger causes immune activation resulting in inflammatory symptoms. GWAS data show an association between multiple genetic polymorphisms (HLA-B51, ERAP1, IL10 and IL23R-IL12RB2) and increased susceptibility to BD. Bacteria as streptococci, an unbalanced microbiome or molecular mimicry trigger the inflammation in BD. Increased production or responsiveness of pro-inflammatory components of the innate immune response (TLR, neutrophils, NK-cells or γδ T-cells) to these triggers may be a crucial step in the pathogenesis of BD. Additionally to an increased autoinflammatory response there is evidence of a dysregulated adaptive immune system, with a disturbed Th1/Th2 balance, expansion of Th17 cells and possibly a decrease in regulatory T cells, resulting in a surplus in pro-inflammatory cytokines. The inflammation causes a typical clinical phenotype including orogenital ulcerations, uveitis and skin lesions. Treatment is aimed at the aberrations found in the innate (neutrophils and γδ-T cells) and adaptive immune system (TNF-α, INF-γ, IL-1), directed at organ involvement and individualized based on patient characteristics.
CONCLUSION
We presented an extensive review into the pathogenesis and treatment options of BD.
Topics: Aminopeptidases; Behcet Syndrome; HLA-B51 Antigen; Humans; Inflammation; Minor Histocompatibility Antigens; Tumor Necrosis Factor-alpha; Uveitis
PubMed: 35038644
DOI: 10.1016/j.semarthrit.2022.151956 -
Arthritis Research & Therapy Oct 2022Clustering is an important clinical feature of Behçet's syndrome (BS) and may have pathogenetic and therapeutic implications. Recent and previous studies on BS...
Clustering is an important clinical feature of Behçet's syndrome (BS) and may have pathogenetic and therapeutic implications. Recent and previous studies on BS phenotype differ substantially in terms of methodology. Correlation matrices and factor analyses were not efficient enough to uncover clusters. Clustering patterns may change according to demographic factors such as age and sex. Clustering patterns may also be profoundly influenced by the misperception of symptoms that are assumed to be secondary to BS, when, in fact, they represent manifestations of BD mimics. This can give rise to misleading conclusions and should be kept in mind when interpreting data obtained by clustering or other phenotype analyses of BS. A true geographical/racial variability in disease expression could be studied in a multinational consensus cohort. Pathogenetic studies in separate clusters of BS have still been lacking.
Topics: Humans; Behcet Syndrome; Cluster Analysis; Phenotype
PubMed: 36309718
DOI: 10.1186/s13075-022-02937-0 -
Clinical and Experimental Rheumatology Sep 2022
Topics: Behcet Syndrome; Humans
PubMed: 36062752
DOI: 10.55563/clinexprheumatol/2eggnf -
Experimental Eye Research Jan 2020Uveitis is usually considered as a vision-threatening multiple system intraocular inflammatory disease. Among uveitis, Vogt-Koyanagi-Harada (VKH) disease and Behcet's... (Review)
Review
Uveitis is usually considered as a vision-threatening multiple system intraocular inflammatory disease. Among uveitis, Vogt-Koyanagi-Harada (VKH) disease and Behcet's disease (BD) are common non-infectious uveitis entities. Although the exact pathogenesis of uveitis is not yet clear, it is acknowledged that the combination of a certain genetic or epigenetic factors with an imbalance in the regulation of the immune response leads to the development of this disease. HLA genes show a strong association with both VKH disease (HLA-DR4, DRB1/DQA1) and BD (HLA-B51) in multiple ethnic populations. Candidate association studies based on a pathogenesis hypothesis laid the foundation for genetic research of uveitis and identified a large number of genes associated with VKH disease or BD including SUMO4, MCP-1, and CTLA4. Genome-wide association study (GWAS) provided a powerful tool for genome-wide level analysis to explore the genetic predisposition for uveitis and revealed several genes to be associated with uveitis including IL23R/C1orf141, STAT4 and ADO/ZNF365/EGR2. Another variant type, the so called copy number variants (CNV), in IL17F, IL23A and C4A also showed an association with uveitis. Additionally, epigenetic factors such as DNA methylation and ncRNAs play important roles in the development of uveitis. The application of new technologies such as whole exome sequencing and whole genome sequencing and other epigenetic modifications such as N6-methyl-adenosine (m6A) modification of mRNAs will be helpful to discover new pathogenic risk genes for uveitis. The understanding of the genetic and epigenetic mechanisms in uveitis may provide a foundation to find novel targets and to develop new strategies in the treatment of uveitis in the near future.
Topics: Behcet Syndrome; DNA Copy Number Variations; DNA Methylation; Epigenesis, Genetic; Female; Genetic Predisposition to Disease; Genome-Wide Association Study; HLA Antigens; Humans; Male; RNA, Untranslated; Uveitis; Uveomeningoencephalitic Syndrome
PubMed: 31669406
DOI: 10.1016/j.exer.2019.107853 -
Current Dermatology Reports 2022Neutrophilic dermatoses are defined by the presence of a sterile neutrophilic infiltrate on histopathology. This review focuses on the pathogenesis, epidemiology,... (Review)
Review
PURPOSE OF REVIEW
Neutrophilic dermatoses are defined by the presence of a sterile neutrophilic infiltrate on histopathology. This review focuses on the pathogenesis, epidemiology, clinicopathological features, diagnosis, and management of four disorders: Sweet syndrome, pyoderma gangrenosum, Behçet syndrome, and neutrophilic eccrine hidradenitis.
RECENT FINDINGS
Recent studies have provided insight into the complex pathogenesis of neutrophilic dermatoses. Evidence supports an intricate interplay of abnormal neutrophil function and inflammasome activation, malignant transformation into dermal infiltrating neutrophils, and genetic predisposition.
SUMMARY
Neutrophilic dermatoses have diverse cutaneous and extracutaneous manifestations and may be associated with significant morbidity and mortality. Common underlying associations include infectious, inflammatory, and neoplastic disorders, as well as drug reactions. Emerging diagnostic and therapeutic frameworks identify an expanding role for biologic and targeted anti-inflammatory therapies.
PubMed: 35310367
DOI: 10.1007/s13671-022-00355-8